[Surgical treatment for 68 patients with Caroli's disease]

To retrospectively investigate the diagnosis and the outcome of Caroli's disease treated by surgical procedures.

The clinical data of 68 patients with Caroli's disease treated by surgical procedures between 1996 and 2002 were reviewed, retrospectively.

The patients, with a M/F ratio of 1:1.35 and a mean age of 46, presented mainly with recurrent cholangitis. Of all the patients, 26 had a history of operation for cholelithiasis or cholangitis. On admission, the image investigations suggested that the lesions located at left lobe in 44 patients, right lobe in 9 patients, and whole liver in 15 patients. The coexisting cyst in common bile duct was found in 20 patients. The malignant transformation was found in 5 patients (8.8%). Hepatectomy was performed in 82.4% of patients, with a morbidity rate of 15.0% and mortality rate of 0 after the surgery. The long-term outcome of symptom-free in hepatectomy group was 90.2%, significantly higher than the 33.3% in non-hepatectomy group (P < 0.01) after a 3 to 10 years of follow-up.

Hepatectomy offers a curative procedure for local Caroli's disease, and liver transplantation is a good option for diffuse sufferers.